CASE REPORTS
JOURNAL ARTICLE
RESEARCH SUPPORT, NON-U.S. GOV'T
Add like
Add dislike
Add to saved papers

Geographic chorioretinal atrophy in pseudoxanthoma elasticum.

PURPOSE: To describe a series of patients with geographic atrophy independent of choroidal neovascularization (CNV) in pseudoxanthoma elasticum and to report progression over time.

DESIGN: Retrospective observational case series.

METHODS: Records of all Vanderbilt Eye Institute patients with pseudoxanthoma elasticum and at least 1 set of color fundus photographs were reviewed (41 eyes of 21 patients). Fluorescein angiography, fundus autofluorescence, and optical coherence tomography images were reviewed, when available. In patients with geographic atrophy and at least 1 year of follow-up, atrophy was measured using fundus photographs. Main outcome measures included incidence of geographic atrophy, progression over time, and macular features associated with development or progression of geographic atrophy.

RESULTS: Eight eyes (20%) of 5 patients had geographic atrophy independent of CNV. Progression was documented in 6 eyes of 4 patients followed for at least 1 year (mean 3.5 years). Mean initial and final area was 2.9 and 9.5 mm(2), respectively, and growth rate was 1.7 mm(2) per year. Of the 6 eyes, 3 had a final visual acuity of 20/20 and the other 3 ranged from 20/150 to 20/400. All 8 eyes had pattern dystrophy, and 5 had linear pigment deposits that appeared to predict development or growth of atrophy.

CONCLUSIONS: Isolated geographic atrophy independent of CNV can develop in pseudoxanthoma elasticum, causing significant vision loss. Linear pigmented pattern dystrophy appears to predate geographic atrophy. Progression is similar to age-related macular degeneration. Recognition of this feature is important, especially if therapies to slow or reverse geographic atrophy become available.

Full text links

We have located links that may give you full text access.
Can't access the paper?
Try logging in through your university/institutional subscription. For a smoother one-click institutional access experience, please use our mobile app.

For the best experience, use the Read mobile app

Mobile app image

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app

All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

By using this service, you agree to our terms of use and privacy policy.

Your Privacy Choices Toggle icon

You can now claim free CME credits for this literature searchClaim now

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app